Extramammary Paget's Disease Resistant to Surgery and Imiquimod Monotherapy But Responsive to Imiquimod Combination Topical Chemotherapy With 5-Fluorouracil and Retinoic Acid: A Case Report

Extramammary Paget's Disease Resistant to Surgery and Imiquimod Monotherapy But Responsive to Imiquimod Combination Topical Chemotherapy With 5-Fluorouracil and Retinoic Acid: A Case Report

Extramammary Paget's disease (EMPD) is an uncommon skin neoplasm that usually affects the elderly population and occurs in the genital, anorectal, or axillary areas. The recommended treatment of EMPD involves surgical excision, including Mohs micrographic surgery; however, surgery is associated with a high rate of recurrence. There have been reports of successful treatment of recurrence with monochemotherapy involving topical imiquimod 5% cream. We report a case of EMPD recurrence after surgery that was resistant to imiquimod monotherapy but that completely resolved after imiquimod was combined with topical 5-fluorouracil (5-FU) and retinoic acid. To our knowledge, this is the first reported case of imiquimod combination therapy with 5-FU and retinoic acid for the treatment of recurrent EMPD.

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Prognosis and Management of Extramammary Paget’s Disease and the Association with Secondary Malignancies

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Prognosis and Management of Extramammary Paget’s Disease and the Association with Secondary Malignancies

Male-to-female ratio was 4:29, and median age was 70 years. Median followup was 68 months, and no patient died from EMPD. The lesion was predominantly found on the vulva (76%). Patch-like, nonconfluent growth was present in 45% of patients, and no patient had pathologic lymph nodes. The most common signs and symptoms were irritation or pruritus (73%) and rash (61%). The presence of patches, invasive tumor growth, or a second malignancy were significantly associated with a higher recurrence rate. The type of operation, either local excision or hemivulvectomy, was not related to the time to recurrence. Complete gross resection was achieved in 94% of cases. Fifty-six percent of patients had microscopically positive margin and this correlated with a significantly higher recurrence rate (p 􏰀 0.002). The tumor recurred clinically in 14 of 33 patients (42%) after a median of 152 months (range 5 to 209 months). In those patients, between one and six reexcisions were performed. In 14 of 33 patients with EMPD (42%), 16 concurrent secondary malignancies were found. Overall survival rates for EMPD patients were similar to those of the general population.

Mammary and extramammary Paget's disease

Mammary and extramammary Paget's disease

Mammary and extramammary Paget’s disease are uncommon intraepithelial ad- enocarcinomas. Both conditions have similar clinical features, which mimic inflammatory and infective diseases. His- tological diagnostic confusion can arise between Paget’s disease and other neo- plastic conditions aVecting the skin, with the most common diVerential diagnoses being malignant melanoma and atypical squamous disease. The glandular diVer- entiation of both mammary Paget’s dis- ease and extramammary Paget’s disease is indicated by morphological appear- ances, the presence of intracellular mucin in many cases, and positive immunohisto- chemical staining for glandular cytokerat- ins, epithelial membrane antigen, and carcinoembryonic antigen. This article provides an overview of mammary and extramammary Paget’s disease and dis- cusses recent evidence regarding the cell of origin. The concepts of primary and secondary Paget’s disease are presented and the diVerential diagnosis is discussed with reference to immunohistochemical markers that might be of diagnostic value. 

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Perianal extramammary paget's disease associated with primary linitis plastica of the rectum: Report of a case

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Perianal extramammary paget's disease associated with primary linitis plastica of the rectum: Report of a case

We report herein a case of perianal extramammary Paget's disease associated with primary linitis plastica of the rectum. An 82-year-old woman was admitted to our hospital for investigation and treatment of a perianal eczematous lesion. A skin biopsy of the lesion revealed perianal extramammary Paget's disease and a barium enema demonstrated diffuse narrowing with an irregular contour at the ampulla recti. Under a suspected diagnosis of linitis plastica of the rectum, an abdominoperineal resection was performed to resect both the rectal and perianal lesions with regional lymphadenectomy. Grossly, marked narrowing and wall thickness were observed at the lower rectum, and a histological diagnosis of signet ring cell carcinoma was confirmed. The perianal eczematous lesion revealed many atypical cells with clear cytoplasm, being Paget cells, throughout the entire epidermis. Sparse distributions of signet ring cells were also observed in the subcutaneous tissue beneath the perianal eczematous lesion. The pathogenesis of perianal extramammary Paget's disease in this patient was therefore considered to be an intraepidermal extension of primary linitis plastica of the rectum.

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Ultrastructural study of extramammary Paget's disease -- histologically showing transition from bowenoid pattern to Paget's disease pattern.

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Ultrastructural study of extramammary Paget's disease -- histologically showing transition from bowenoid pattern to Paget's disease pattern.

Histological, immunohistochemical, and ultrastructural studies were performed on two cases of histologically unusual extramammary Paget's disease. Histologically, the central area of the lesions showed a bowenoid pattern, and the peripheral area showed typical extramammary Paget's disease. The transition zone showed an intermediate pattern. All these areas were positive for CEA and EMA, and negative for S-100 protein. Ultrastructurally, in the intermediate pattern, the tumour cells had abundant cytoplasmic glycogen, and the widened intercellular spaces contained numerous glycogen particles, which were probably secreted by the tumour cells. It is well known that eccrine glands, but not apocrine glands, secrete glycogen particles. Therefore, the present findings suggest that some cases of extramammary Paget's disease are a proliferation of germinative cells with eccrine gland differentiation.

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